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高嗜酸性粒细胞综合征

Hypereosinophilic Syndrome.

作者信息

Curtis Casey, Ogbogu Princess

机构信息

Section of Allergy and Immunology, Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, Wexner Medical Center at the Ohio State University, Rm. 220, 2012 Kenny Rd., Columbus, OH, 43221, USA.

出版信息

Clin Rev Allergy Immunol. 2016 Apr;50(2):240-51. doi: 10.1007/s12016-015-8506-7.

Abstract

Hypereosinophilic syndrome consists of a group of disorders characterized by abnormal accumulation of eosinophils in the blood or peripheral tissues, independent of known secondary causes of eosinophilia such as parasitic infection. Clinical manifestations of the condition are highly variable, ranging from asymptomatic eosinophilia to severe tissue damage and end-organ failure. This entity has been recognized for decades, with early studies identifying distinct groups of patients with differing symptoms, exam findings, laboratory abnormalities, and prognosis. In the past, these patients were treated with non-targeted immunosuppressive agents, often with limited efficacy. More recently, advances in the knowledge of eosinophil biology and molecular diagnostics have allowed for more specific delineation of the many disease subgroups that characterize hypereosinophilic syndrome. Identification of these groups has led to a personalized management approach to the condition, with improved diagnostic techniques as well as stratification of patients into more effective treatment groups. This review will discuss the evolution of the definition of hypereosinophilic syndrome, outline current disease classifications, provide a guide for evaluation and monitoring, and discuss current and future therapeutic modalities.

摘要

高嗜酸性粒细胞综合征由一组疾病组成,其特征是血液或外周组织中嗜酸性粒细胞异常积聚,且与已知的嗜酸性粒细胞增多的继发原因(如寄生虫感染)无关。该病症的临床表现高度可变,从无症状的嗜酸性粒细胞增多到严重的组织损伤和终末器官衰竭。这个实体已经被认识了几十年,早期研究确定了具有不同症状、检查结果、实验室异常和预后的不同患者群体。过去,这些患者接受非靶向免疫抑制剂治疗,疗效往往有限。最近,嗜酸性粒细胞生物学和分子诊断知识的进展使得能够更具体地划分高嗜酸性粒细胞综合征的许多疾病亚组。对这些亚组的识别导致了针对该病症的个性化管理方法,包括改进诊断技术以及将患者分层到更有效的治疗组。本综述将讨论高嗜酸性粒细胞综合征定义的演变,概述当前的疾病分类,提供评估和监测指南,并讨论当前和未来的治疗方式。

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